Gangliogliomas (GGs) represent approximately 0.4%-1.0% of all brain tumors. Anaplastic gangliogliomas (AGGs) form 5-10% of all GGs. They are a mixed neuronal-glial tumor of central nervous system and composed by two cell lines; neuronal (ganglionic) and glial cells. Anaplastic component of AGGs is usually glial cells. Malignant neuronal component is a rare condition. Here we report an 8-year-old male patient who was diagnosed with primary AGG with malignant neuronal component and was treated with surgery, adjuvant radiotherapy and chemotherapy.